Endocrine involvement during the course of GPA is exceptional, and only a few sporadic observations were reported. Thus the exact meaning of endocrinopathies associated with this vasculitis is not yet well known. Fortuitous association or specific localization of vasculitis in the endocrine glands? The purpose of this minireview is to study the endocrine involvement in granulomatosis with polyangeiitis and to discuss its possible pathogenic mechanisms.
Introduction: Ciclosporin A is an immunosuppressive molecule widely used in Behçet's disease (BD), especially for severe ocular and/or neurological disorders. Its particular toxicity in this field has, however, largely limited its use during this vasculitis. We report an aspect.Case report: 26-year-old patient followed since the age of 20 for BD with severe ocular involvement (bilateral posterior steroid-resistant uveitis with a significant decrease in visual acuity) requiring treatment with Ciclosporin A at a dose of 5mg/kg/twice daily during the first month and then 5mg/kg/day as a maintenance dose to control uveitis. The one-year control report noted a creatinine at 147μmol/l with aseptic leucocyturia at 400 elements/l. blood pressure was normal and there was no associated hematuria or proteinuria. The infectious and toxic investigation was normal. The ultrasound showed two kidneys of normal size and echo-structure. Renal biopsy revealed acute interstitial nephritis with images of toxic tubular necrosis. Cyclosporine was discontinued and replaced with azathioprine. Renal function normalized three weeks after stopping the molecule.
Conclusion:periodic and regular monitoring of renal function (blood and urine tests) is strongly recommended in order to detect renal damage early in the BD; especially if Ciclosporin A treatment.
Introduction: Cutaneous lesions are frequent and very polymorphic in diabetic subjects. However, the combination of authentic acquired ichthyosis(AI) with diabetes mellitus (DM) remains exceptional and unusual. We report an original case of AI in type 2 DM.Case Report: 55-years-old patient, known diabetic type 2 for 15 years, and treated with oral antidiabetic drugs, was hospitalized for recent significant weight loss (15 kg in two weeks) with appearance of diffuse and very annoyingskin lesions.
Physical examination and skin biopsy confirmed the diagnosis of AI. His diabetes was very unbalanced withHbA1C at 16%. The advanced investigations had eliminated an underlying autoimmune disease, infection, cancer, and hematological malignancies. After treatment with insulinanalogs in intensive protocol, the evolution was favorable with normalization of the glycemic parameters and progressive disappearance of the cutaneous lesions.
Conclusion:The association of AI with DM remains exceptional and only two cases are found in the literature. Our observation is, to our knowledge, the third reporting this association. The occurrence of AI in a type 2 diabetic may mark the need to introduce insulin.
Introduction: Kikuchi-Fujimoto's disease (KFD) or histiocytic necrotizing lymphadenitis is a rare clinico-pathological entity of unknown etiology that primarily affects young Asian women. The association of this disease with dys-immune systemic disease is rare. We report two cases.Observation 1: 28 year old woman, having systemic lupus erythematosus (SLE) with cutaneous, hematologic, and renal complication, who developed six years after cervical and axillary febrile lymphadenopathy. A lymph node biopsy showed histological and Immunohistochemical aspects suggesting KFD.Observation 2: 30 year old woman, without medical history was hospitalized for febrile superficial poly -lymphadenopathy. Etiological investigation was negative. Lymph node biopsy confirmed the diagnosis of KFD. Two years later primary Sjogren's syndrome was diagnosed in this patient.Favorable evolution was noted in both cases after 1-2 months.
Conclusion:KFD is a very rare histological diagnosis. Underlying autoimmune conditions are rarely reported; particularly the association with primary Sjogren's syndrome is exceptional. These associations represent a real diagnostic challenge and they deserve to be known by physicians.
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